However the basal cell carcinoma also can reveal eccrine differentiation rarely 2 especially in adenoid basal cell carcinoma variation. For language access assistance, contact the ncats public information officer. It usually spreads to regional lymph nodes, with liver, lungs, and bones being the most common sites of distant metastasis. The main differential diagnosis for this neoplasm is metastatic mucinous adenocarcinoma. Trichilemmal carcinoma also has clear cells, but lacks eccrine ducts and may show trichohyalin granules. A 76yearold woman presented with an asymptomatic papule on her nose that had been present for one year. Eccrine and squamous differentiation in merkel cell.
She is diseasefree 14 months after initial diagnosis. Eccrine carcinoma is the most common subtype of adnexal. Skin adnexal tumors sat are rare tumors that make up about 1%2% of all cutaneous malignancies. Cureus eccrine porocarcinoma with zosteriform metastasis. Syringomatous carcinoma of the scalp presenting as a slowly enlarging patch of alopecia. Most common sites for this tumor are eyelid and periocular tissues. Primary mucinous eccrine carcinoma of the skin a rare clinical tumor with many differential diagnoses.
Metastatic eccrine porocarcinoma annals of oncology. Eccrine carcinomas of the eyelid are rare and comprise three types. Periumbilical region is a rare site for this neoplasm. Eccrine porocarcinoma usually are found in adults older than 60 and appear as a single red elevated solid mass nodule or a thickened or elevated lesion plaque. Primary cutaneous mucinous carcinoma with extramammary. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 toll free. Two lesions occurred on the upper eyelid and 2 on the cheek. Primary mucinous eccrine carcinoma pmec is a rare skin adnexal neoplasm.
Ductal eccrine or apocrine adenocarcinomas tend to show a more prominent and exclusive tubular or cribriform growth pattern than porocarcinomas. The low prevalence rate and limited literature on eccrine carcinoma ec pose a. Syringoid eccrine carcinoma is a rare sweat gland carcinoma that can be difficult to diagnose. They affect the eccrine sweat glands, which are the major sweat glands of the body. It is a carcinoma with high metastatic and recurrent potential. Squamoid eccrine ductal carcinoma is a cutaneous malignancy that originates from the eccrine sweat gland. Primary cutaneous mucinous carcinoma with extramammary paget. Squamoid eccrine ductal carcinoma the dermatologist.
A case of canine apocrine sweat gland adenoma, clear cell. It mainly occurs in the elderly, with equal incidence in both sexes. Perianal eccrine adenocarcinoma world journal of surgical. Eccrine porocarcinoma of the ear mimicking basaloid. Eccrine carcinoma ecc, first described in 1865, is a rare skin tumor accounting for less than 0. Pdf eccrine carcinoma is a quite rare malignant tumor that typically arises from a normal sweat gland and that features a rather high recurrence rate. May 26, 2014 other tumors with eccrine differentiation are malignant cylindroma, malignant eccrine poroma, malignant eccrine spiradenoma, malignant nodular hidradenoma and malignant chondroid syringoma.
Eccrine porocarcinoma an overview sciencedirect topics. Dermoscopy showed pseudocysts, irregular linear crown vessels, and yellowishbrown globules surrounded by white halos. Mohs micrographic surgery for squamoid eccrine ductal. Eccrine and squamous differentiation in merkel cell carcinom. Oral capecitabine achieves response in metastatic eccrine. The primary treatment of choice is surgical wide local excision of the tumour with histological confirmation of tumour free margins 3, 4, 8, although mohs micro. Pdf metastatic syringoid eccrine carcinoma of the nipple. Sections of eccrine carcinoma show a tumour composed of basaloid cells infiltrating the dermis figure 1. Eccrine sweat gland carcinoma of the eyelid with orbital. Eccrine porocarcinoma is a rare malignant tumor that develops in the eccrine glands, appearing as a primary tumor, or by malignant transformation of an eccrine poroma. Chemotherapy of rare skin adnexal tumors anticancer research. Squamoid eccrine ductal carcinoma is rare, with as few as cases reported in the literature. Among them, eccrine porocarcinoma is the most frequent, with an aggressive behavior.
Electron microscopy performed on one case showed cytoplasmic densecore neurosecretory granules and intercellular lumina lined by cells containing microvilli. Eccrine carcinoma is a rare cutaneous malignancy of the sweat glands with an aggressive potential and high risk for metastasis and recurrence. Tumors arising from sweat glands represent approxi mately 1% of all primary skin lesions. A clinicopathologic study was made of 35 patients with primary eccrine adenocarcinoma diagnosed in the past 20 years from among 450,000 consecutive skin biopsy specimens.
Subungual syringoid eccrine carcinoma of the great toe nail complex a case report john f. The antibody used did not stain the innermost are of the tumor, where cells may be negative for ck. A 10year disease free survival rate of 56% in the absence of lymph node. We represent a rare case report of clear cell eccrine carcinoma with comedonecrosis in the neck region which is an unusual. Oct 09, 2012 apocrine eccrine carcinomas are rare and associated with poor prognosis. A large study carried by busam and coworkers evaluated the staining pattern of primary cutaneous sweat gland carcinoma and primary or metastatic breast carcinoma 4. Carsten christof boedeker, md otolaryngologyhead and neck surgery 2006 5. Recently, two series of 7 and 30 cases, respectively, were.
Squamoid eccrine ductal carcinoma sedc is a rare malignant neoplasm of the skin that arises from the eccrine gland and presents on histology with a squamoid appearance, potentially mistaken for squamous cell carcinoma. We report a case of syringoid eccrine carcinoma occurring on the scalp of a 66yearold woman and analyzed its cytokeratin expression immunohistochemically to clarify its histogenesis. In addition, we discuss features that differentiate. Metastatic eccrine carcinoma with stomach and pericardial. Basal cell carcinoma with eccrine differentiation is an invasive, slowgrowing tumor that usually occurs on the scalp. Clinically, they presented as a slowly growing cyst or swelling. Basal cell carcinoma with eccrine differentiation eccrine. Journal of the college of physicians and surgeons pakistan. Its differentiation toward eccrine structure and function predicts the chronic, recurrent, locally infiltrative clinical course observed in patients to date. Ductal eccrine carcinoma is a rare malignant eccrine sweat gland tumor with ductal differentiation. Two to five million eccrine glands are distributed on almost the entire skin surface, with sparing of the lips, external auditory canal, glans penis, labia minora, and clitoris. Mar 01, 2005 the golden retriever is a breed that shows a predisposition to such tumors. The clinical and histologic appearance is often nonspecific. Alerts and notices synopsis eccrine gland carcinoma encompasses a rare group of sweat gland neoplasms that can be subdivided into many different types based on a wide histological spectrum.
Mucinous eccrine carcinoma mec is a rare malignant tumor that typically arises in the periorbital area. Pdf cutaneous vulvar carcinomas are predominantly of squamous cell carcinoma type. The mass was sent for histopathology and after confirming tumor free margins, lid reconstruction was done by procuring a glabellar flap. Sec is a rare type of sweat gland carcinoma originally described as basal cell carcinoma with eccrine differentiation eccrine epithelioma by freeman and winkelmann in 1969. Given its rather poor response to adjuvant therapy, wide excision of the lesion with tumor free margins may offer a reasonable chance for longterm control of this neoplasm. Whole genome and whole transcriptome genomic profiling of a. Petct imaging of squamoid eccrine ductal carcinoma. The most common site of occurrence was the lower eyelid 8 cases. Overall, eccrine adenocarcinomas are destructive lesions with a tendency to local recurrence. There are a few case reports documenting the diagnosis and management of eccrine carcinoma. Eccrine carcinoma is an extremely rare malignant skin cancer arising from eccrine sweat glands with a high metastatic potential. The histopathologic findings include basaloid epithelial elements, cystic spaces, and a fibrous or myxomatous stroma.
Eccrine carcinoma is a dermalbased tumor composed of infiltrating ductsglands associated with a sclerotic stroma. This case study presents an unusual case of what we believe is a lowgrade eccrine carcinoma showing squamous differentiation. First described as eccrine epithelioma by freeman and winkelmann in 1969,7 sec have also been reported under many different labels, including eccrine carcinoma, squamoid eccrine ductal carcinoma, sclerosing sweat duct carcinoma. May 05, 2016 the combined light microscopic and phenotypic profile led to a diagnosis of a squamoid eccrine ductal carcinoma sedc. To our knowledge, this is the second case of vulvar sweat gland carcinoma of eccrine origin associated with. This result made this antibody unreliable in differentiating both entities. Mucinous eccrine carcinoma of the eyelid snow 1992. A case of eccrine porocarcinoma, located in the breast.
Pdf basal cell tumor with eccrine differentiation eccrine. It is frequently found on the lower extremities, and it equally affects both sexes in the sixth and seventh decade. We present a case of a 22yearold woman with a presentation of syringoid eccrine carcinoma in the subungual region of the hallux. Apocrine eccrine carcinomas are rare and associated with poor prognosis. Syringoid eccrine carcinoma is an extremely rare cutaneous malignant tumor, thought to be derived from eccrine sweat apparatus.
F18 fdg petct imaging of eccrine sweat gland carcinoma of. Histologically, they were wellcircumscribed, typically multinodular tumors with solid or partially cystic nodules, frequently showing areas of. The tumor ultimately extended deeper into the orbit and invaded the sclera, the optic nerve sheath, and the ethmoidal. The enigmatic eccrine epithelioma eccrine syringomatous carcinoma am j dermatopathol. Eccrine carcinoma is a rare tumour that typically presents as a slow growing plaques or nodules on the scalp but can arise at other sites. Histopathologies closely associated with ec include hidroadenocarcinoma, spiradenocarcinoma, and porocarcinoma. These ultrastructural and immunohistochemical features support the concept of eccrine differentiation in these. The authors report the 73rd case of primary mec of the skin. The patient described in this report demonstrated epithelial elements with marked similarity to eccrine ducts. Chemotherapeutic drugs that selectively target cancerpromoting pathways may complement conventional therapeutic approaches. The clinical importance of sedc lies in its locoregional aggressiveness and metastatic potential. Routine histopathology was diagnostic of eccrine acrospiroma with malignant transformation. Eccrine carcinoma tumors are locally aggressive with a high rate of recurrence. Eccrine carcinoma ec is an appendageal cancer that originates from the eccrine sweat gland and accounts for an estimated 0.
Eccrine porocarcinoma of the external ear sage journals. Eccrine carcinoma an overview sciencedirect topics. Eccrine carcinoma is a quite rare malignant tumor that typically arises from a normal sweat gland and that features a rather high recurrence rate subsequent to simple excision. Eccrine porocarcinoma of the ear mimicking basaloid squamous cell carcinoma dr. Eccrine porocarcinoma is a rare type of skin cancer that may occur by chance or may develop from a benign noncancerous skin tumor. A 70yearold man had a sweat gland carcinoma in his right upper eyelid that was slowly growing, but it clinically remained confined to the lid tissues for a period of 25 years. However, studies on genetic alterations and egfr and her2 status of apocrine eccrine carcinomas are few in number. May 09, 2018 h an average age of 70 years range, 4884 years.
May 30, 2019 several eccrine carcinoma subtypes bear histologic resemblance to a welldescribed benign counterpart. Neither preoperative staging nor lymphadenectomy had been performed. Eccrine porocarcinoma shares dermoscopic characteristics with eccrine poroma. Eccrine mucinous carcinoma genetic and rare diseases. Therefore, immunohistochemistry is often helpful with making the diagnosis. Metastatic work up in the patient was within normal limits. Characteristics and survival of patients with eccrine carcinoma.
Introductionsyringoid eccrine carcinoma sec is an extremely rare malignant adnexal tumor of eccrine origin, first described by freeman and wilkemann in 1969. Lack of reliable immunohistochemical markers and similarity to other. Subungual syringoid eccrine carcinoma of the great toe. Feb 04, 20 eccrine carcinoma is an extremely rare malignancy of the skin with few well documented cases reported in the literature. Syringoid eccrine carcinoma sec is an extremely rare malignant adnexal tumour of eccrine origin. For patients with followup data available, metastasis free survival.
Cutaneous apocrine gland carcinoma, a subtype of sweat gland carcinoma, is a very. If you have problems viewing pdf files, download the latest version of adobe reader. Histologically the following four distinct variants were identified. Resection margins were free, but lymphovascular invasion was present. Eccrine epithelioma is a rare tumor that may be mistaken for syringoma, basal cell carcinoma, adenocystic carcinoma, sweat gland carcinoma, or metastatic tumor. Eccrine carcinoma is a rare skin condition characterized by a plaque or nodule on the scalp, trunk, or extremities 669 it originates from the eccrine sweat glands of the skin, accounting for less than 0. They are less likely to show solid poroid areas or components with squamous differentiation. Eccrine porocarcinoma of the ear mimicking basaloid squamous. We report a case of syringoid eccrine carcinoma occurring on the scalp of a 66yearold woman and analyzed its cytokeratin expression immunohistochemically to clarify its.
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